OBJECTIVES: To investigate the ubiquinone-10 content in lymphocytes from phenylketonuric patients. DESIGN AND METHODS: We compared 23 patients with 25 age-matched controls. Ubiquinone-10 was analyzed by HPLC with electrochemical detection. RESULTS: Ubiquinone-10 concentrations were significantly lower in patients (77-270 nmol/g of protein) compared with controls (190-550) (p < 0.001). Significantly negative correlation was observed between ubiquinone-10 and phenylalanine (r = -0.441; p < 0.05). CONCLUSIONS: Ubiquinone-10 concentrations are decreased in lymphocytes from phenylketonuric patients. This deficiency is associated with high plasma phenylalanine concentrations.
OBJECTIVES: To investigate the ubiquinone-10 content in lymphocytes from phenylketonuricpatients. DESIGN AND METHODS: We compared 23 patients with 25 age-matched controls. Ubiquinone-10 was analyzed by HPLC with electrochemical detection. RESULTS:Ubiquinone-10 concentrations were significantly lower in patients (77-270 nmol/g of protein) compared with controls (190-550) (p < 0.001). Significantly negative correlation was observed between ubiquinone-10 and phenylalanine (r = -0.441; p < 0.05). CONCLUSIONS:Ubiquinone-10 concentrations are decreased in lymphocytes from phenylketonuricpatients. This deficiency is associated with high plasma phenylalanine concentrations.
Authors: Karolina M Stepien; Robert Heaton; Scott Rankin; Alex Murphy; James Bentley; Darren Sexton; Iain P Hargreaves Journal: J Clin Med Date: 2017-07-19 Impact factor: 4.241